A LARGE multicentre cohort study has found that hypopigmented mycosis fungoides (HMF) in adults generally follows an indolent clinical course, with most patients presenting with early-stage disease and responding well to skin-directed therapies.
Insights from a Multicentre Hypopigmented Mycosis Fungoides Study
The retrospective study analysed 224 adults with biopsy-confirmed HMF treated across six US tertiary referral centres between 2011 and 2023. The median age at diagnosis was 44 years, and most patients were Black (83%) and female (73%). Nearly all patients (98%) presented with early-stage disease.
HMF is an uncommon variant of mycosis fungoides characterised by hypopigmented skin lesions, but data on its clinical behaviour in adults have been limited. In this cohort, 71% of patients presented with hypopigmented lesions alone, while 29% had mixed-variant disease. Outcomes did not differ significantly between these groups.
Among 141 patients with available immunophenotyping data, CD8-positive predominance was observed in 63%. However, neither immunophenotype nor mixed-variant presentation appeared to influence disease progression or overall outcomes.
Of the 207 patients who received treatment, 90% were managed with skin-directed therapies alone. Response data were available for 198 patients, showing a complete response in 26%, partial response in 51%, stable disease in 17%, and progressive disease in 6%.
After a median follow-up of 39 months, stage progression occurred in only 14 patients (6%). Importantly, progression from early-stage to advanced-stage disease was rare, affecting just five of 219 patients (2%) who initially presented with early-stage HMF.
The investigators also evaluated T-cell receptor (TCR) gene rearrangements. Peripheral blood TCR monoclonality was detected in 27% of tested patients and was associated with poorer treatment response. However, it was not linked to an increased risk of disease progression.
Favourable Outcomes in Adult Hypopigmented Mycosis Fungoides
Overall, the findings suggest that adult HMF has a favourable prognosis, with low rates of stage progression and good responses to skin-directed treatment. The authors found no independent baseline clinical or pathological factors associated with disease progression, highlighting the generally benign course of this rare mycosis fungoides subtype in adults.
Reference
Park JB et al. Clinicopathologic Features and Long-Term Outcomes of Adult Hypopigmented Mycosis Fungoides. JAMA Dermatol. 2026; DOI: 10.1001/jamadermatol.2026.1793.
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